• Marianne Kambouchner, Jean-François Emile, Marie-Christine Copin, Aurore Coulomb-Lherminé, Jean-Christophe Sabourin, Valeria Della Valle, Chiara Sileo, Hubert Ducou Le Pointe, Hugues Bégueret, Louise Galmiche, Anne Lambilliotte, François Paraf, Marjorie Piche, Christophe Piguet, Anne Rullier, Véronique Secq, Isabelle Serre, Jean-François Bernaudin, and Jean Donadieu.
• Pathology Department, APHP, Centre Hospitalier Universitaire (CHU) Avicenne, 93000 Bobigny, France. Electronic address: marianne.kambouchner@aphp.fr.
• Hum. Pathol. 2019 Jul 1; 89: 51-61.

AbstractChildhood pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease. Its pulmonary histopathology, according to comprehensive clinical-radiological findings and BRAFV600E mutation status, has not yet been thoroughly documented. From the 167 childhood PLCH cases entered in the French National Histiocytosis Registry (1983-2016), we retrieved lung biopsies from a consecutive retrospective series of 17 patients, diagnosed when they were 2 weeks to 16 years old (median, 9.4 years), and report the clinical and histopathological findings herein. Histological analyses of biopsies (16 surgical and 1 postmortem) found the following features, alone or associated: Langerhans cell (LC) nodules with cavitation (9/17), cysts (14/17), fibrotic scars (2/17), peribronchiolar topographic distribution of the lesions (10/17), and accessory changes, like stretch emphysema (7/17). Those characteristics closely resemble those describing adult PLCH. However, unusual findings observed were 2 large nodules and a diffuse interstitial LC infiltrate. BRAFV600E mutation was detected in 4 of 12 samples tested, notably in the 3 with unusual features. In conclusion, childhood PLCH mostly shares the common histology features already described in adult PLCH, regardless of age. Because smoking is considered the major trigger in PLCH pathogenesis, the findings based on this series suggest other inducers of bronchiolar LC recruitment, especially in very young patients.Copyright © 2019 Elsevier Inc. All rights reserved.

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